Lesson 1_Hematopoietic System

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Questions and Answers

Which characteristic distinguishes blood from water?

  • Higher density (correct)
  • Lower viscosity
  • Lighter consistency
  • Lesser weight

What proportion of total body weight does blood constitute?

  • Approximately 2%
  • Approximately 20%
  • Approximately 15%
  • Approximately 8% (correct)

Which of the following is NOT a primary function of blood?

  • Transporting oxygen and carbon dioxide
  • Generating nerve impulses (correct)
  • Regulating pH balance
  • Regulating body temperature

Following centrifugation of a blood sample, which layer contains the smallest volume?

<p>Buffy coat (C)</p> Signup and view all the answers

Which of the following is a key component of blood plasma that plays a crucial role in blood clotting?

<p>Fibrinogen (A)</p> Signup and view all the answers

Which characteristic is exclusive to white blood cells when compared to red blood cells?

<p>Presence of a nucleus (A)</p> Signup and view all the answers

What is the primary role of platelets in the bloodstream?

<p>Initiating blood clotting (D)</p> Signup and view all the answers

A pluripotent stem cell differentiates into either a myeloid stem cell or a lymphoid stem cell. What is the PRIMARY difference in the maturation process of these two cell types?

<p>Lymphoid stem cells mature in the lymphatic tissue, while myeloid stem cells mature in the bone marrow. (C)</p> Signup and view all the answers

During hemopoiesis, a monoblast differentiates into a monocyte. At what point in this differentiation process does the cell become irreversibly committed to becoming a monocyte, losing its ability to differentiate into other types of blood cells?

<p>When it progresses to a progenitor cell. (D)</p> Signup and view all the answers

Consider a scenario where a patient's blood sample is centrifuged, and the plasma layer appears abnormally turbid and has a noticeably elevated concentration of lipids. Which of the following functional impairments is MOST likely to be observed in this patient, based solely on this information?

<p>Inefficient thermoregulation as a result of altered blood viscosity and flow dynamics. (A)</p> Signup and view all the answers

Blood is more viscous than water.

<p>True (A)</p> Signup and view all the answers

Plasma constitutes approximately 75% of the total blood volume.

<p>False (B)</p> Signup and view all the answers

Erythrocytes, also known as white blood cells, are the most dense and account for around 45% of blood volume.

<p>False (B)</p> Signup and view all the answers

The buffy coat, primarily composed of erythrocytes and granulocytes, constitutes approximately 25% of blood volume.

<p>False (B)</p> Signup and view all the answers

Progenitor cells, originating from myeloid and lymphoid stem cells within bone marrow, retain the capacity for self-replication indefinitely, thereby maintaining a constant reserve of undifferentiated hemopoietic precursors throughout the lifespan.

<p>False (B)</p> Signup and view all the answers

Which hemopoietic growth factor primarily stimulates the production of red blood cells?

<p>Erythropoietin (EPO) (B)</p> Signup and view all the answers

What is the primary function of thrombopoietin (TPO)?

<p>Regulating the growth and maturation of megakaryocytes (A)</p> Signup and view all the answers

Why do mature erythrocytes appear as biconcave discs?

<p>Due to the absence of a nucleus and certain organelles. (A)</p> Signup and view all the answers

Which of the following occurs during the transformation of erythroblasts to reticulocytes?

<p>Expulsion of the nucleus (B)</p> Signup and view all the answers

What is the role of ferroxidase in iron transport?

<p>It oxidizes ferrous iron to ferric iron for binding to transferrin. (D)</p> Signup and view all the answers

Which characteristic of methemoglobin distinguishes it from normal hemoglobin?

<p>Methemoglobin has a reduced ability to bind oxygen due to iron being in the Fe3+ (ferric) state. (B)</p> Signup and view all the answers

What physiological response is triggered by decreased erythropoiesis due to low oxygen levels?

<p>Temporary syncope due to lack of oxygen in the brain (D)</p> Signup and view all the answers

A patient presents with cyanosis, fatigue, and dizziness. Lab results reveal a methemoglobin level of 8%. What is the MOST likely underlying condition?

<p>Mild methemoglobinemia (A)</p> Signup and view all the answers

Which of the following represents the correct sequence of erythropoiesis, starting from a pluripotent hematopoietic stem cell?

<p>Proerythroblast → Erythroblast → Reticulocyte → Erythrocyte (A)</p> Signup and view all the answers

A researcher is investigating novel therapeutic targets to enhance oxygen delivery in patients with severe anemia. Targeting which of the following processes would MOST directly increase the oxygen-carrying capacity of erythrocytes, assuming all other physiological conditions remain constant?

<p>Modulating the concentration of 2,3-diphosphoglycerate (2,3-DPG) within erythrocytes to optimize hemoglobin's oxygen affinity. (B)</p> Signup and view all the answers

A male with an erythrocyte count of 5.6 million per microliter is within the typical range.

<p>False (B)</p> Signup and view all the answers

During erythropoiesis, the erythroblast differentiates directly into a reticulocyte after gaining a nucleus.

<p>False (B)</p> Signup and view all the answers

Decreased erythropoiesis leads to hypertension by increasing blood viscosity.

<p>False (B)</p> Signup and view all the answers

Administration of ferroxidase inhibitors could theoretically reverse the effects of methemoglobinemia in severe cases.

<p>False (B)</p> Signup and view all the answers

What does a reticulocyte count primarily indicate?

<p>The rate of red blood cell production. (B)</p> Signup and view all the answers

A patient's hematocrit is reported as 32%. According to the provided information, which condition is most likely?

<p>Anemia (A)</p> Signup and view all the answers

What is the clinical significance of Mean Corpuscular Volume (MCV) in routine blood analysis?

<p>Provides an estimate of the average size of red blood cells. (C)</p> Signup and view all the answers

If a patient's lab results show a Mean Corpuscular Hemoglobin (MCH) value outside the normal range (27-31 picograms/cell), what aspect of the red blood cells is directly affected?

<p>Hemoglobin amount (A)</p> Signup and view all the answers

Increased Red Blood Cell Distribution Width (RDW) is associated with which condition?

<p>Anisocytosis (A)</p> Signup and view all the answers

What does a hyperchromic result in Mean Corpuscular Hemoglobin Concentration (MCHC) indicate about red blood cells?

<p>Higher than normal hemoglobin concentration (A)</p> Signup and view all the answers

Which characteristic distinguishes poikilocytosis from anisocytosis?

<p>Poikilocytosis involves variations in cell shape, while anisocytosis involves variations in cell size. (D)</p> Signup and view all the answers

Why is Erythrocyte Sedimentation Rate (ESR) considered a screening test rather than a diagnostic test?

<p>ESR is affected by numerous factors and cannot specifically identify a disease. (A)</p> Signup and view all the answers

In cases of suspected anemia, which combination of laboratory results would MOST strongly suggest a diagnosis of microcytic hypochromic anemia?

<p>Decreased MCV, decreased MCHC, elevated RDW (A)</p> Signup and view all the answers

A patient presents with a constellation of symptoms suggestive of chronic inflammation. Their ESR is significantly elevated, but routine blood tests, including complete blood count and differential, are within normal limits. Which of the following laboratory tests would MOST likely provide additional, specific information to pinpoint the underlying inflammatory condition?

<p>C-reactive protein (CRP) and rheumatoid factor (RF) assays. (C)</p> Signup and view all the answers

Anemia in general is defined based on cell size (MCV) 📏 and amount of hemoglobin (MCH) 💉

<p>True (A)</p> Signup and view all the answers

  1. What does the RDW test measure?

<p>The variation in RBC size (D)</p> Signup and view all the answers

True or False: A normal RDW range is 10.5% –True or False: A normal RDW range is 10.5% – 20%. 20%.

<p>False (B)</p> Signup and view all the answers

An increased RDW value (anisocytosis) may indicate

<p>Wide variation in RBC sizes (C)</p> Signup and view all the answers

Which of the following is NOT a use of the ESR test?

<p>Diagnosing a specific disease (B)</p> Signup and view all the answers

The normal ESR value for males is <20 mm/hr, and for females, it is <30 mm/hr.

<p>True (A)</p> Signup and view all the answers

Why is the blood pregnancy test used less often than the urine test?

<p>A. It is more expensive and requires a lab test.</p> <p>(A)</p> Signup and view all the answers

What is the underlying cause of the fatigue experienced in anemia?

<p>Lack of oxygen needed for ATP production. (A)</p> Signup and view all the answers

Which of the following is a common cause of iron-deficiency anemia?

<p>Inadequate absorption or excessive loss of iron. (A)</p> Signup and view all the answers

What is the primary cause of pernicious anemia?

<p>Inability of the stomach to produce intrinsic factor. (D)</p> Signup and view all the answers

Which type of anemia results from excessive blood loss?

<p>Hemorrhagic anemia. (D)</p> Signup and view all the answers

What is the underlying mechanism in hemolytic anemia?

<p>Premature rupture of red blood cells. (D)</p> Signup and view all the answers

What immunological process underlies Hemolytic Disease of the Newborn (HDN)?

<p>Maternal antibodies attacking fetal red blood cells. (D)</p> Signup and view all the answers

What is the rationale for administering Rh Immunoglobulin (RhoGAM) to Rh-negative mothers?

<p>To prevent the Rh-negative mother from becoming sensitized to Rh-positive fetal red blood cells. (D)</p> Signup and view all the answers

Which condition is characterized by abnormal fluid accumulation in two or more fetal compartments?

<p>Hydrops fetalis. (A)</p> Signup and view all the answers

What is the primary defect in thalassemias?

<p>Abnormal hemoglobin production due to genetic mutations. (B)</p> Signup and view all the answers

In beta thalassemia, which globin chains are affected?

<p>Beta chains. (A)</p> Signup and view all the answers

What is the underlying cause of aplastic anemia?

<p>Decreased or failed production of all types of blood cells in the bone marrow. (D)</p> Signup and view all the answers

What is the genetic basis of sickle cell anemia?

<p>Mutation in the globin gene, leading to abnormal hemoglobin. (A)</p> Signup and view all the answers

In sickle cell anemia, what is the consequence of the altered shape of red blood cells?

<p>Easy rupture and obstruction of blood vessels. (B)</p> Signup and view all the answers

What is the predominant type of hemoglobin in healthy adults?

<p>HbA/HbA1. (A)</p> Signup and view all the answers

Which of the following characterizes HbSS in sickle cell anemia?

<p>A mutation in both β-globin chains, exhibiting normal symptoms of anemia. (D)</p> Signup and view all the answers

What is the primary consequence of polycythemia?

<p>Increased blood viscosity, increased BP, and thrombosis. (D)</p> Signup and view all the answers

A patient presents with fatigue, paleness, and intolerance to cold. Which laboratory test would be MOST relevant to initially evaluate for a potential diagnosis of anemia?

<p>Complete blood count (CBC). (A)</p> Signup and view all the answers

A researcher is investigating the mechanism by which sickle cell trait (HbAS) confers resistance to malaria. Which of the following cellular changes is MOST directly responsible for killing malarial parasites in HbAS erythrocytes?

<p>Altered plasma membrane causing potassium leakage and low potassium levels. (C)</p> Signup and view all the answers

In α thalassemia, a condition known as hydrops fetalis occurs when all four genes responsible for alpha chain synthesis are defective. What is the primary reason this is fatal?

<p>Inability to synthesize HbF (fetal hemoglobin) needed for fetal oxygen transport. (A)</p> Signup and view all the answers

A patient is diagnosed with anemia due to chronic kidney disease. Which of the following mechanisms is the MOST likely cause of the anemia in this patient?

<p>Decreased erythropoietin production. (A)</p> Signup and view all the answers

A patient with sickle cell anemia experiences frequent vaso-occlusive crises. Which of the following interventions would be MOST effective in preventing these crises?

<p>Hydroxyurea therapy. (A)</p> Signup and view all the answers

A patient is suspected of having beta thalassemia minor. What would the genetic analysis MOST likely reveal?

<p>Defect in one beta-globin gene. (D)</p> Signup and view all the answers

A researcher discovers a novel mutation in the gene encoding ferroportin, a protein responsible for iron export from cells. Which of the following hematological conditions would MOST likely result from this mutation?

<p>Hemochromatosis due to iron accumulation in cells. (B)</p> Signup and view all the answers

A patient presents with a history of frequent blood transfusions due to thalassemia major. Which complication is of GREATEST concern due to the increased red blood cell turnover and transfusion frequency?

<p>Iron overload leading to organ damage. (D)</p> Signup and view all the answers

A hematopoietic stem cell acquires a mutation that impairs its response to erythropoietin. Given the pathophysiology of erythropoiesis, which of the following downstream effects would be MOST likely?

<p>Decreased production of erythrocytes, leading to anemia. (C)</p> Signup and view all the answers

Which laboratory test is used to determine red blood cell size in anemia classification?

<p>Mean Corpuscular Volume (MCV) (B)</p> Signup and view all the answers

In settings of sufficient iron availability, which metabolic adaptation is MOST critical for sustaining red blood cell production during chronic anemia?

<p>Increased synthesis of 2,3-diphosphoglycerate (2,3-DPG) (A)</p> Signup and view all the answers

What is the most common symptom associated with anemia?

<p>Fatigue (D)</p> Signup and view all the answers

Anemia can cause intolerance to cold due to reduced ATP and heat production; is this statement true or false?

<p>True (A)</p> Signup and view all the answers

Which condition is NOT a direct cause of anemia?

<p>Elevated blood glucose levels (B)</p> Signup and view all the answers

What is the primary deficiency that directly leads to pernicious anemia?

<p>Intrinsic factor (A)</p> Signup and view all the answers

Pernicious anemia is classified as what type of anemia based on red blood cell size?

<p>Macrocytic (D)</p> Signup and view all the answers

Which cells in the gastric mucosa are directly responsible for secreting intrinsic factor?

<p>Parietal cells (A)</p> Signup and view all the answers

Where does the absorption of the Vitamin B12-Intrinsic Factor complex primarily occur after it leaves the stomach?

<p>Ileum (C)</p> Signup and view all the answers

A patient with a history of autoimmune disorders develops pernicious anemia. Which pathophysiological mechanism is MOST likely contributing to their condition?

<p>Antibody-mediated destruction of parietal cells. (D)</p> Signup and view all the answers

What is the primary cause of hemorrhagic anemia?

<p>Excessive loss of RBCs through bleeding (A)</p> Signup and view all the answers

Which of the following statements accurately describes chronic hemorrhagic anemia?

<p>It involves gradual blood loss over time. (C)</p> Signup and view all the answers

Which of the following etiologies is MOST commonly associated with hemolytic anemia?

<p>Blood transfusion reaction (A)</p> Signup and view all the answers

Which factor is LEAST likely to contribute to the development of hemolytic anemia?

<p>Increased production of erythropoietin (B)</p> Signup and view all the answers

In which type of anemia does red blood cell lysis occur prematurely, diminishing their typical lifespan?

<p>Hemolytic anemia (C)</p> Signup and view all the answers

What is the underlying cause of Beta (β) Thalassemia?

<p>A defect or absence of beta-globin chain synthesis (D)</p> Signup and view all the answers

Which statement about Beta Thalassemia Major is accurate?

<p>Two genes are defective, leading to severe anemia. (B)</p> Signup and view all the answers

How many genes are responsible for the production of alpha-globin chains, and therefore implicated in cases of Alpha Thalassemia?

<p>4 (D)</p> Signup and view all the answers

Which of the following statements BEST describes the outcome of Hydrops Fetalis?

<p>It results from all four defective alpha genes and is typically fatal. (C)</p> Signup and view all the answers

Which characteristic is exclusive to leukocytes (white blood cells) compared to other blood components?

<p>Presence of a nucleus (B)</p> Signup and view all the answers

What cellular component is absent in mature white blood cells?

<p>Hemoglobin (C)</p> Signup and view all the answers

Which of the following is a primary function of leukocytes that distinguishes them from erythrocytes?

<p>Immune response (D)</p> Signup and view all the answers

Which of the following is a characteristic feature of leukocytes that distinguishes them from erythrocytes?

<p>Presence of a nucleus (C)</p> Signup and view all the answers

Which of the following is present in leukocytes but absent in erythrocytes?

<p>Nucleus (C)</p> Signup and view all the answers

What structural feature allows leukocytes to migrate out of blood vessels and into surrounding tissues?

<p>Ameboid movement (C)</p> Signup and view all the answers

In contrast to erythrocytes, which primarily function within blood vessels, how do leukocytes typically perform their functions?

<p>Within tissues outside the bloodstream (A)</p> Signup and view all the answers

What property of white blood cells enables them to leave capillaries and enter the interstitial fluid?

<p>Diapedesis (D)</p> Signup and view all the answers

Which cellular feature allows leukocytes to actively target and engulf pathogens, a function absent in erythrocytes?

<p>Phagocytic ability (D)</p> Signup and view all the answers

What is a key function that differentiates leukocytes from erythrocytes, enabling them to elicit immune responses?

<p>Antigen recognition (B)</p> Signup and view all the answers

What is the most accurate description of leukocytes compared to other blood cells?

<p>The only complete cells in blood (D)</p> Signup and view all the answers

Which structural characteristic distinguishes leukocytes as 'complete cells' within the bloodstream?

<p>Presence of a nucleus (D)</p> Signup and view all the answers

In what significant way do white blood cells differ structurally from red blood cells?

<p>White blood cells have a defined nucleus (D)</p> Signup and view all the answers

Unlike other blood cells, leukocytes possess a specific attribute that significantly contributes to their immunological functions. What is this attribute?

<p>Capability of diapedesis (B)</p> Signup and view all the answers

What significance does the presence of a nucleus in leukocytes have for their function, compared to erythrocytes?

<p>Enables protein synthesis for immune responses (B)</p> Signup and view all the answers

Under normal physiological conditions, approximately what percentage of whole blood volume is comprised of leukocytes?

<p>Less than 1% (B)</p> Signup and view all the answers

Which blood component makes up the smallest percentage of blood volume?

<p>Leukocytes (C)</p> Signup and view all the answers

In a standard complete blood count (CBC), what is the typical percentage range of leukocytes in relation to the total blood volume?

<p>Less than 1% (D)</p> Signup and view all the answers

Which of the following best represents the proportion of leukocytes within total blood volume, highlighting their role in immune responses rather than volume contribution?

<p>Typically less than 1% of blood volume (A)</p> Signup and view all the answers

When considering the total blood volume, which of the following accurately describes the relative abundance of leukocytes?

<p>They make up a small fraction, generally less than 1%. (A)</p> Signup and view all the answers

Leukocytes are the only complete cells in the blood; considering blood composition, how does this impact their oxygen-carrying capacity compared to erythrocytes?

<p>Leukocytes do not transport oxygen. (A)</p> Signup and view all the answers

A researcher is developing a novel drug that specifically targets the process of diapedesis. Which cell type would be MOST directly affected by this drug?

<p>Leukocytes (D)</p> Signup and view all the answers

A pathologist observes a blood smear and notes the absence of nuclei in the majority of cells. Which functionality is MOST likely compromised in these cells?

<p>Antibody production (D)</p> Signup and view all the answers

A novel microfluidic device is designed to isolate leukocytes from whole blood based on a structural difference. If the device successfully separates cells lacking hemoglobin, which of the following cell types is MOST likely being targeted?

<p>Leukocytes (A)</p> Signup and view all the answers

Imagine a hypothetical scenario where erythrocytes retained their nuclei throughout their maturation. What would be the MOST SIGNIFICANT direct consequence of this alteration on erythrocyte function?

<p>Compromised oxygen-carrying capacity (C)</p> Signup and view all the answers

Which virus is the causative agent of infectious mononucleosis (IM)?

<p>Epstein-Barr Virus (EBV) (D)</p> Signup and view all the answers

In infectious mononucleosis, activated B lymphocytes morphologically resemble which type of blood cell, lending to the disease's name?

<p>Monocytes (B)</p> Signup and view all the answers

Which clinical manifestation is least likely to be associated with infectious mononucleosis?

<p>Persistent hypotension with bradycardia (A)</p> Signup and view all the answers

What is the MOST accurate statement regarding treatment strategies for infectious mononucleosis?

<p>Treatment is primarily supportive, focusing on symptom management, as there is no specific antiviral cure. (B)</p> Signup and view all the answers

A 19-year-old college student presents with fatigue, fever, sore throat, and significant swelling in the cervical lymph nodes. Initial tests suggest infectious mononucleosis. Which of the following complications, while rare, would warrant IMMEDIATE and aggressive intervention?

<p>Splenic rupture following a minor trauma (B)</p> Signup and view all the answers

What is the immediate, initial response to a blood vessel injury during hemostasis?

<p>Vascular spasm to reduce blood flow. (C)</p> Signup and view all the answers

Which of the following best describes the role of thromboxane A2 (TXA₂) in hemostasis?

<p>Stimulating the activation and aggregation of additional platelets. (C)</p> Signup and view all the answers

What enzymatic conversion directly leads to a stabilized blood clot during the coagulation cascade?

<p>Fibrinogen to fibrin. (A)</p> Signup and view all the answers

Which statement accurately contrasts the initiation mechanisms of the intrinsic and extrinsic coagulation pathways?

<p>The intrinsic pathway is activated by subendothelial collagen exposure, while the extrinsic pathway is initiated by the release of tissue factor from damaged cells. (C)</p> Signup and view all the answers

If a drug inhibits platelet adhesion but not platelet activation, which of the following stages of hemostasis would be MOST directly affected?

<p>The initial formation of pseudopodia and their binding to the exposed collagen. (A)</p> Signup and view all the answers

Match the following coagulation factors with their corresponding names:

<p>I = Fibrinogen II = Prothrombin III = Tissue Factor / Thromboplastin IV = Calcium Ions V = Proaccelerin VII = Proconvertin VIII = Antihemophilic factor A IX = Antihemophilic factor B X = Stuart factor XI = Antihemophilic factor C XII = Hageman factor XIII = Fibrin-stabilizing factors</p> Signup and view all the answers

Match the following substances with their actions:

<p>Alpha-2-macroglobulin = Inactivates thrombin and plasmin Prostacyclin (PGI2) = Powerful inhibitor of platelet adhesion and release Antithrombin III (AT-III) = Blocks action of factors II, IX, X, XI, and XII Protein C = Inactivates factors V and VIII; enhances activity of plasminogen activators Alpha-1-antitrypsin = Inhibits factor XI Heparin = Combines with AT-III, increasing its effectiveness in blocking thrombin</p> Signup and view all the answers

What is the primary mechanism by which plasmin contributes to hemostatic control?

<p>Digesting fibrin threads to dissolve clots. (B)</p> Signup and view all the answers

Which biological role is NOT associated with prostacyclin (PGI2)?

<p>Promotion of platelet adhesion. (B)</p> Signup and view all the answers

Which coagulation factor is NOT directly blocked by antithrombin III (AT-III)?

<p>Factor VII (A)</p> Signup and view all the answers

What is the primary difference between anticoagulation and antiplatelet mechanisms in antithrombotic drugs?

<p>Anticoagulants slow down the clotting mechanism and reduce fibrin formation, while antiplatelets prevent platelet aggregation. (D)</p> Signup and view all the answers

What is the primary mechanism of action of thrombolytic agents such as streptokinase?

<p>Dissolving existing blood clots. (D)</p> Signup and view all the answers

Which of the following is a characteristic symptom of hemophilia?

<p>Joint bleeding. (B)</p> Signup and view all the answers

What is the key distinction between a thrombus and an embolus?

<p>A thrombus is a stationary clot, while an embolus is a dislodged and transported clot. (B)</p> Signup and view all the answers

What is the MOST common inherited bleeding disorder?

<p>Von Willebrand's disease. (D)</p> Signup and view all the answers

In Von Willebrand's Disease, what factor is deficient?

<p>Factor VIII and VWF (A)</p> Signup and view all the answers

Which cell type is primarily affected in multiple myeloma?

<p>Plasma cells. (A)</p> Signup and view all the answers

How does heparin exert its anticoagulant effects?

<p>Enhancing the activity of antithrombin III. (C)</p> Signup and view all the answers

A patient is diagnosed with Thrombocytopenia, what does this mean?

<p>Abnormally decreased platelet count (C)</p> Signup and view all the answers

Why are individuals with Von Willebrand disease at a higher risk of bleeding complications?

<p>Because VWF is essential for platelet adhesion and Factor VIII stability. (C)</p> Signup and view all the answers

Considering the mechanisms of hemostasis, which factor would MOST directly counteract the effects of thromboxane A2 (TXA2)?

<p>Prostacyclin (PGI2). (C)</p> Signup and view all the answers

A researcher is investigating a novel drug that selectively inhibits alpha-2-macroglobulin. Which of the following processes would be MOST directly affected by this drug?

<p>Inactivation of thrombin and plasmin. (D)</p> Signup and view all the answers

Match the hemostatic disorders with their descriptions:

<p>Thrombocytosis = An abnormal increase in the platelet count Thrombocytopenia = An abnormal decrease in the platelet count Hemophilia = Deficiency or absence of a blood clotting factor Intravascular Clots = Stationary clot that forms in a blood vessel Von Willebrand's Disease = Deficient in VWF and Factor VIII Multiple Myeloma = Cancerous disorder of plasma cells</p> Signup and view all the answers

Flashcards

Blood

The only liquid connective tissue in the body, heavier, thicker, and more viscous than water. Transports nutrients, regulates temperature and pH.

Centrifugation of Blood

Separation technique to identify blood components by density. Reveals plasma, buffy coat, and erythrocytes.

Plasma

The liquid component of blood, makes up 55% of blood volume, contains water, proteins (albumin, globulins, fibrinogen), electrolytes, gases, nutrients, enzymes, hormones, waste products.

Hemopoiesis

Formation, development, and maturation of blood elements.

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Red Blood Cells (Erythrocytes)

Transport oxygen from lungs to tissues and carbon dioxide from tissues to lungs. Biconcave, anucleated, and red due to hemoglobin.

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White Blood Cells (Leukocytes)

Contribute to the immune system by defending the body against infections. Possess a nucleus, lack hemoglobin, and are larger than RBCs.

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Platelets

Plays a crucial role in blood clotting by forming a plug at the site of vascular injury. Small, anucleated cell fragments.

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Pluripotent Stem Cell (Hemocytoblast)

Immature, undifferentiated cell in bone marrow that develops into myeloid or lymphoid stem cells.

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Myeloid Stem Cell

Develop in red bone marrow and differentiate into progenitor cells, then precursor cells, ultimately forming RBCs and platelets.

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Lymphoid Stem Cell

Begin development in red bone marrow, complete it in lymphatic tissue, and develop into lymphocytes.

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Buffy Coat

The smallest layer of blood after centrifugation, comprising about 1% of the total volume, it contains platelets and leukocytes.

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Progenitor Cells

Cells that arise from myeloid stem cells, lose the ability to self-reproduce, and are destined to differentiate into specific blood elements like RBCs and platelets.

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Precursor Cell (-blast)

An immature blood cell, identified by the suffix '-blast', that develops into formed elements; for example, a monoblast develops into a monocyte.

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Erythropoietin (EPO)

Stimulates red blood cell production in the red bone marrow.

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Thrombopoietin (TPO)

Facilitates the growth and maturation of megakaryocytes, which produce platelets.

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Cytokines

Regulate the maturation of blood cells within the bone marrow.

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Erythropoiesis

The process of red blood cell production.

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Reticulocyte

Cell directly before erythrocyte.

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Transferrin

Iron (Fe2+) transported in blood stream via transferrin.

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Methemoglobin

Hemoglobin that has been oxidized (Fe2+ → Fe3+).

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Methemoglobinemia

Condition with higher-than-normal methemoglobin levels.

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Erythrocytes (RBCs)

Red blood cells; anucleated, biconcave discs containing hemoglobin to transport oxygen and carbon dioxide.

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Erythropoiesis Stages

Process of red blood cell development: Pluripotent stem cell → proerythroblast → erythroblast → reticulocyte → erythrocyte.

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Hemoglobin Functions

Oxygen binds to hemoglobin; carbon dioxide binds with hemoglobin; maintains blood pH; releases nitric oxide for proper blood flow.

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Iron Transport by Transferrin

Iron transported in blood via transferrin, oxidized by ferroxidase to the ferric state for transport, made in the liver, has highest affinity for iron.

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Reticulocyte Count

Measures the percentage of reticulocytes in the blood to determine the rate of erythropoiesis.

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Hematocrit

The percentage of red blood cells in the total blood volume.

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Mean Corpuscular Volume (MCV)

The average size of a single red blood cell, measured in femtoliters (fl).

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Mean Cell Hemoglobin (MCH)

The average amount of hemoglobin present in a single red blood cell

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Mean Corpuscular Hemoglobin Concentration (MCHC)

The concentration of hemoglobin within a given volume of red blood cells.

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Red Blood Cell Distribution Width (RDW)

Measures variation in red blood cell size, used to diagnose and classify anemias.

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Anisocytosis

Irregularity in size of RBCs shown in the blood sample.

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Poikilocytosis

Variation in shape of the red cell population.

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Erythrocyte Sedimentation Rate (ESR)

Measures the rate at which red blood cells settle in a tube over time.

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Pregnancy Test

Blood test that measures human chronic gonadotropin (hCG).

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Anemia

Reduced oxygen-carrying capacity of blood, often classified by cell size (microcytic, macrocytic, normocytic) and hemoglobin amount.

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MCV Test

Laboratory test to determine the average size of red blood cells.

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MCH Test

Laboratory test to determine the average amount of hemoglobin in a red blood cell.

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Iron-Deficiency Anemia

Anemia caused by inadequate iron absorption or excessive iron loss, resulting in small, pale red blood cells.

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Pernicious Anemia

Anemia caused by the stomach's inability to produce intrinsic factor, leading to vitamin B12 deficiency and impaired RBC production.

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Hemorrhagic Anemia

Anemia caused by excessive loss of red blood cells through bleeding, either acute (sudden) or chronic (prolonged).

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Hemolytic Anemia

Anemia caused by premature rupture of red blood cells due to inherent defects, toxins, parasites, or incompatible blood transfusions.

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Hydrops Fetalis

Condition where abnormal fluid accumulates in fetal compartments, often due to Rh incompatibility (HDN) or non-immune causes.

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Thalassemias

Genetic blood disorders with abnormal hemoglobin production, leading to microcytic, hypochromic, short-lived red blood cells.

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Beta (𝛃) Thalassemia

Thalassemia where synthesis of beta globin chains is defective or missing.

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Alpha (𝛂) Thalassemia

Thalassemia where synthesis of alpha globin chains is defective or missing.

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Aplastic Anemia

Rare blood disorder where bone marrow fails to produce all types of blood cells (RBCs, WBCs, platelets).

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Sickle Cell Anemia

Abnormal hemoglobin (Hgb-S) causes red blood cells to become stiff and sickle-shaped, leading to hemolysis and vascular obstruction.

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Polycythemia

Abnormal increase in circulating red blood cells, increasing blood viscosity, blood pressure, and thrombosis risk.

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Pancytopenia

Condition characterized by a significant reduction in the number of all three major types of blood cells (RBCs, WBCs, and platelets).

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Anemia Definition

A condition characterized by a reduction in the blood's capacity to transport oxygen, often due to a decreased number of red blood cells or hemoglobin.

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Fatigue in Anemia

A common symptom of anemia resulting from reduced oxygen delivery to tissues, causing a feeling of tiredness and lack of energy.

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Oxygen-Carrying Capacity and Anemia

Reduced oxygen-carrying capacity of the blood.

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Causes of Anemia

High blood glucose levels are not a direct cause of anemia; anemia is directly caused by reduced RBCs, decreased hemoglobin, and low oxygen-carrying capacity.

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Pernicious Anemia Cause

Pernicious anemia primarily results from a deficiency in intrinsic factor (IF), hindering vitamin B12 absorption.

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Pernicious Anemia: Macrocytic

Pernicious anemia is characterized as a macrocytic (megaloblastic) anemia, meaning red blood cells are larger than normal.

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Intrinsic Factor Source

The stomach is responsible for producing intrinsic factor; dysfunction leads to intrinsic factor deficiency and pernicious anemia.

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Vitamin B12 Absorption Site

Vitamin B12 absorption primarily occurs in the small intestine, not the stomach, where intrinsic factor is produced.

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Pernicious Anemia Causes

Liver disease and high iron intake are NOT common causes of pernicious anemia; typical causes include Vitamin B12 deficiency, and malabsorption.

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Hemorrhagic Anemia Cause

Anemia due to excessive blood loss, either sudden (acute) or prolonged (chronic).

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Acute Hemorrhagic Anemia

Sudden blood loss, such as from a major injury or surgery.

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Chronic Hemorrhagic Anemia

Ongoing, persistent blood loss, such as from ulcers or heavy menstruation.

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Causes of Hemolytic Anemia

Blood transfusion reactions, parasites, toxins or genetic defects that cause RBC destruction.

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Beta (𝛃) Thalassemia Cause

Caused by a defect or absence in the synthesis of the beta-globin chains, leading to varying degrees of anemia depending on the severity of the genetic defect.

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Alpha (𝛂) Thalassemia: Silent Carrier

A mild form of Alpha Thalassemia where individuals have only one defective alpha-globin gene. They typically show no symptoms but can pass the affected gene to their offspring.

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Alpha (𝛂) Thalassemia: Gene Count

Four. There are two alpha-globin genes on each chromosome 16, so a total of four genes control alpha-globin production.

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Leukocytes

Also known as white blood cells; complete cells in the blood that lack hemoglobin.

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What causes IM?

Infectious mononucleosis is caused by the Epstein-Barr Virus.

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Kissing Disease

Infectious mononucleosis is often called the "Kissing Disease" due to its transmission through saliva.

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Why 'mononucleosis'?

Infectious mononucleosis is called "mononucleosis" because it causes an increase in mononuclear lymphocytes in the blood, specifically B lymphocytes that resemble monocytes.

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Cure for IM?

There is no specific cure for infectious mononucleosis; treatment focuses on relieving symptoms like rest and pain management.

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Not a symptom of IM?

High blood pressure is not typically a symptom of infectious mononucleosis.

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Vascular Spasm

Vasoconstriction to reduce blood loss.

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Platelet Plug Purpose

To temporarily seal injuries in small blood vessels.

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Coagulation Cascade Result

Conversion of fibrinogen to fibrin, forming a stable clot.

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TXA₂, Serotonin, ADP Function

Activated platelets release these to recruit and activate more platelets.

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The Intrinsic Pathway

This pathway is initiated by damage within the blood vessel.

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Prostacyclin (PGI2)

A powerful inhibitor of platelet adhesion and release.

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Antithrombin III (AT-III)

Blocks the action of factors II, IX, X, XI, and XII in the coagulation cascade.

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Protein C

Inactivates factors V and VIII and enhances the activity of plasminogen activators for clot breakdown.

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Alpha-2-macroglobulin

Inactivates thrombin and plasmin, preventing clot formation and breakdown.

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Alpha-1-antitrypsin

Inhibits factor XI, preventing activation of the intrinsic coagulation pathway.

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Heparin

Combines with AT-III, enhancing its effectiveness in blocking thrombin and preventing clot formation.

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Fibrinolysis

Dissolves blood clots by digesting fibrin threads and inactivating clotting factors.

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Anticoagulants

Medications that prevent blood clot formation or their activity.

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Activated Protein C (APC)

Inactivates factors V and VIII; enhances plasminogen activators.

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Anticoagulants (AC)

Slows down clotting, reduces fibrin formation.

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Antiplatelet (AP)

Prevents platelet aggregation.

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Thrombolytic Agents

Dissolves existing blood clots.

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Thrombocytosis

An abnormal increase in platelet count.

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Thrombocytopenia

An abnormal decrease in platelet count.

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Hemophilia

Deficiency of a blood clotting factor; sex-linked, inherited.

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Hemophilia A

Deficiency of Factor VIII.

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Hemophilia B

Deficiency of Factor IX.

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Thrombus

A stationary clot formed in a blood vessel.

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Emboli

Dislodged thrombi transported elsewhere.

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Study Notes

  • Blood is the only liquid connective tissue.
  • Blood is heavier, thicker, and more viscous than water.
  • Water makes up a larger percentage of the body's liquid volume.
  • Cells are 60-90% water by weight.
  • Blood is about 8% of total body weight.

Functions of Blood

  • Transports oxygen, carbon dioxide, metabolic wastes, and nutrients.
  • Regulates body temperature and pH via buffer systems and hemoglobin.
  • Maintains pH of blood.
  • Releases nitric oxide (NO) causing vasodilation.

Blood Components

  • Separated via centrifugation by density.
  • Plasma is the top, least dense layer
  • Buffy coat is a small layer (1% of blood) with platelets and leukocytes.
  • Erythrocytes are the most dense layer (~45% of blood), also known as red blood cells.

Plasma

  • Makes up about 55% of total blood volume.
  • It is a yellowish liquid.
  • Consists of 91.5% water and 8.5% solutes (proteins).
  • Proteins (albumin, globulins, fibrinogen) are produced by hepatocytes.
  • Fibrinogen is essential for blood clotting and wound healing.
  • Antibodies and immunoglobulins contribute to immunity.
  • Includes electrolytes, nutrients, enzymes, and hormones.
  • Contains waste products like urea, ammonia, and metabolites.

Formed Elements

  • Hemopoiesis is the formation, development, and maturation of blood's formed elements.

Hemopoietic Growth Factors

  • Erythropoietin (EPO) stimulates RBC production (erythropoiesis) in red bone marrow.
    • Used to treat anemia, chronic kidney disease, and chemotherapy-induced anemia.
  • Thrombopoietin (TPO) regulates megakaryocyte growth and maturation.
    • Used for patients with low platelet counts.
  • Cytokines regulate blood cell maturation in the bone marrow.

Red Blood Cells (Erythrocytes)

  • Transports oxygen from the lungs to tissues.
  • Carries carbon dioxide from tissues back to the lungs.
  • Biconcave shape and lacks a nucleus (anucleated).
  • Red due to hemoglobin.
  • Normal values:
    • Male: 5.4 million per µL
    • Female: 4.8 million per µL

Erythropoiesis

  • RBC production from pluripotent hematopoietic stem cells differentiating into proerythroblasts (pronormoblasts), then erythroblasts (normoblasts).
  • Erythroblasts lose their nucleus and organelles to form reticulocytes.
  • Reticulocytes are transported to the blood.
  • Reticulocytes become erythrocytes 1-2 days after release from the red bone marrow.
  • Loss of the nucleus leads to the biconcave shape.

Hemoglobin

  • Gives RBCs red color.
  • Each RBC contains hundreds of billions of hemoglobin molecules.
  • Each hemoglobin molecule contains about 4 heme rings.
  • Normal levels:
    • Male: 13-18 g/dl
    • Female: 12-16 g/dl
  • Transports oxygen and carbon dioxide.
    • Oxygen "enters" hemoglobin.
    • Carbon dioxide only binds with hemoglobin.

Methemoglobin

  • Does not effectively bind oxygen.
  • Normal concentration is less than 2%.
  • High levels cause methemoglobinemia, leading to hypoxia, cyanosis, and death.

Methemoglobinemia

  • Hemoglobin is oxidized to methemoglobin (Fe2+ → Fe3+).
  • Levels exceeding 70% are lethal.
  • Symptoms: cyanosis, fatigue, headache, dizziness.

Iron Transport

  • Iron (ferrous state) transported in blood by binding to transferrin.
  • Ferrous iron is oxidized by ferroxidase to the ferric state.
  • Ferric iron binds with transferrin (made in the liver).
  • Transferrin is a serum protein with the highest affinity for iron, for iron transport.

Negative Feedback

  • Decreased erythropoiesis can lead to temporary syncope or fainting.

White Blood Cells (Leukocytes)

  • Contributes to the immune system.
  • Defends the body against infections and foreign invaders.
  • Possess a nucleus, lack hemoglobin, and are larger than RBCs.
  • Only complete cells in the blood.
  • Do not contain Hgb.

Platelets

  • Platelets play a vital part in blood clotting.
  • Form a plug at the site of vascular injury.
  • Small cell fragments without a nucleus.
  • Platelets release TXA₂ (thromboxane 2), serotonin (5-HT), and ADP to activate more platelets during platelet plug formation.

From Stem Cell

  • Hemopoiesis begins with immature, pluripotent stem cells or hemocytoblasts in the bone marrow.
  • Pluripotent stem cells differentiate into specific cells with distinct responsibilities.
  • Myeloid stem cells start development in the red bone marrow and differentiate into progenitor cells.
  • Progenitor cells cannot reproduce and are committed to forming specific blood elements.
  • Progenitor cells differentiate into precursor cells (-blasts), which then develop into formed elements.
    • For example, monoblasts will develop into monocytes.
  • Lymphoid stem cells begin development in the red bone marrow but complete it in lymphatic tissue.
  • Lymphoid stem cells develop into lymphocytes.

Blood Laboratory Tests

Reticulocyte Count

  • Measures the percentage of reticulocytes in the blood.
  • Determines the rate of erythropoiesis.
  • Normal value ranges from 0.5% to 1.5% of total blood cells.
  • Reticulocytopenia indicates a low count.
  • Reticulocytosis indicates a high count.
  • Abnormal counts may result from bleeding, anemia, kidney disease, or vitamin B deficiency.

Hematocrit

  • Measures the percentage of RBC in the blood.
  • Normal values:
    • Males: 38.8% - 48.6%
    • Females: 35.5% - 44.9%
  • Low count indicates anemia.
  • High count (>65%) indicates polycythemia.

Mean Corpuscular Volume (MCV)

  • Determines the average RBC size in femtoliters (fl).
  • Formula: MCV = (Hematocrit % * 10) / RBC count (millions/mm3).
  • Microcytic: low average size.
  • Normocytic: normal size (80-100 fl).
  • Macrocytic: high average size.

Anisocytosis

  • Condition of variation in RBC size.
  • Blood may have microcytic and macrocytic RBCs.

Mean Cell Hemoglobin (MCH)

  • Measures the average amount of hemoglobin per red blood cell.
  • Formula: MCH = Hemoglobin / RBC count (millions).
  • Normal value ranges from 27 - 31 picograms/cell.

Mean Corpuscular Hemoglobin Concentration (MCHC)

  • Measures the amount of hemoglobin per volume of RBCs.
  • Formula: MCHC = (Hemoglobin (g/dL) * 100) / Hematocrit %.
  • Hypochromic: Low MCHC.
  • Normochromic: Normal MCHC.
  • Hyperchromic: High MCHC, no white in the middle.
  • Normal values range from 32 - 36 grams/dL.
  • Normochromic: normal color, uniform weight.
  • Hypochromic: less red RBCs, more white in the middle.
  • Hyperchromic: very red RBCs, less white in the middle.

Red Blood Cell Distribution Width (RDW)

  • Measures the variation in the size of red blood cells.
  • Assesses the variation in the size of RBCs.
  • Used to diagnose and classify anemias.
  • Normal range: 10.5% - 15%.
  • Increased RDW indicates anisocytosis, or a wide variation in the size of RBCs.
Anisocytosis
  • Irregular distribution of sizes/circumference of RBCs.
  • A condition wherein there is a variation in RBC size.
Poikilocytosis
  • Observed normal variation in shapes of the red cell population.
  • The six types are: Acanthocytes, Codocytes, Dacrocytes, Drepanocytes, Echinocytes, and Elliptocytes.
  • Acanthocytes: have thorn-like projections.
  • Codocytes: have a darkly stained central area.
  • Dacrocytes: are pear-shaped or tear drop-shaped.
  • Drepanocytes: are sickle cells.
  • Echinocytes: have short thorn-like projections.
  • Elliptocytes: are elliptical in shape.

Erythrocyte Sedimentation Rate (ESR)

  • Also known as Sedimentation rate or Sed Rate.
  • Measures the rate at which red blood cells settle in a vertical tube of blood.
  • Does not use a blood centrifuge, since the rate of separation of components is monitored.
  • It is a screening test for inflammatory, autoimmune diseases, or cancer, but it is NOT used to diagnose a disease or disorder.
  • Normal values:
    • Male: less than 20mm/hr
    • Female: less than 30mm/hr.

Pregnancy Test

  • Determines if your blood contains the hormone, human chronic gonadotropin (hCG).
  • This test is more sensitive than urine tests and thus used less often.
  • Can detect pregnancy about six to eight days after ovulation.

Erythrocyte Disorders

Anemias

  • Indicates a reduced oxygen-carrying capacity of blood.
  • Usually defined based on cell size (microcytic, macrocytic, normocytic) and hemoglobin amount.
  • A common symptom is fatigue.
  • Fatigue stems from a lack of oxygen needed for ATP production.
  • Can cause intolerance to cold due to reduced ATP and heat production.
  • Caused by a reduced number of RBCs and or decreased hemoglobin levels.
  • Symptoms include fatigue, intolerance to cold, and paleness.
  • Fatigue and cold intolerance stem from a lack of oxygen needed for ATP production, which generates heat.
  • Mean Corpuscular Volume (MCV) lab test is used to determine RBC size in anemia classification.

Iron-Deficiency Anemia

  • Caused by inadequate iron absorption or excessive loss.
  • Is the most prevalent type of anemia worldwide.
  • Characterized as microcytic, hypochromic anemia.
  • Meat and malunggay are good sources of iron.
  • A normal blood smear would be normocytic, normochromic.
  • Common in vegetarians, pregnant women, those with heavy menstruation, and frequent blood donors.

Pernicious Anemia

  • Caused by a lack of intrinsic factor (IF).
  • Is a macrocytic (megaloblastic) anemia, meaning RBCs are larger than normal.
  • The stomach's dysfunction leads to intrinsic factor deficiency.
  • Vitamin B12 absorption does not occur in the stomach but is needed for RBC production
  • The stomach's inability to produce intrinsic factor (IF) needed for Vitamin B12 (Cobalamin) absorption in the small intestine.
  • Intrinsic Factor (IF) is a glycoprotein produced by parietal cells in the gastric body and fundus,
  • The absence of IF leads to decreased Vitamin B12 absorption, reducing RBC production, leading to pernicious anemia.
  • Macrocytic/Megaloblastic Anemia.
  • Common causes include Vitamin B12 deficiency, liver disease, malabsorption, alcohol use, pernicious anemia, and hypothyroidism.
  • High iron intake is not a common cause.

Hemorrhagic Anemia

  • Excessive loss of RBCs through bleeding.
  • Can be acute or chronic.
  • Acute hemorrhagic anemia is caused by extraordinarily heavy bleeding from large wounds or injuries.
  • Chronic is caused by slow, prolonged bleeding from stomach ulcers.

Hemolytic Anemia

  • Premature rupture of RBCs.
  • Presents with distortions in erythrocyte shape.
  • Causes include inherent defects, toxins, parasites, antibodies from incompatible blood transfusions, and blood transfusion reactions.
  • Hemolytic anemia can be caused by parasites, toxins, and genetic defects.
Hemolytic Disease of the Newborn (HDN)
  • Also called Erythroblastosis fetalis, occurs due to blood type incompatibility between mother and baby.
  • HDN due to Rh incompatibility is more common.
  • Rh factor is a protein on the surface of RBCs; Rh positive indicates its presence, while Rh negative indicates its absence.
  • Autoimmune condition where the mother's antibodies target and lyse the fetus's RBCs.
  • If the baby's blood cells cross into the mother's circulation, the mother's immune system recognizes them as foreign.
  • The mother creates antibodies to attack the foreign blood cells.
  • Complications range from mild to severe.
  • The mother becomes sensitized, and her immune system retains the antibodies.
  • HDN is more likely to occur during the 2nd or subsequent pregnancies or after a miscarriage/abortion.
  • Women with Rh-negative blood are identified early in pregnancy through a blood test.
  • Rh-negative mothers, who have not been sensitized, are given Rh Immunoglobulin (RhoGAM) to prevent their antibodies from reacting to the baby's Rh-positive RBCs.
  • RhoGAM is given around the 28th week of pregnancy and again within 72 hours of giving birth.

Hydrops Fetalis

  • Is a serious, life-threatening condition characterized by abnormal fluid accumulation in two or more fetal compartments, leading to edema.
  • Occurs during pregnancy and presents a high risk to the fetus's health and survival.
  • Severe swelling can interfere with organ function, often resulting in infant death shortly after delivery.
  • Causes include is immune and non-immune causes.
Immune Causes
  • Include Rh incompatibility between the mother and fetus, leading to HDN, and blood group incompatibilities with antibody reactions.
Non-Immune Causes
  • Include genetic disorders, infections (e.g., Parvovirus), structural abnormalities, metabolic disorders, and cardiac abnormalities.

Thalassemias

  • Are a group of genetic blood disorders with abnormal hemoglobin production, leading to anemia.
  • Caused by mutations in genes controlling hemoglobin production.
  • Caused by a defect or absence of genes responsible for alpha- or beta-chain synthesis, leading to premature RBC death.
  • Components synthesis is reduced or absent.
  • Characterized by microcytic, hypochromic, short-lived RBCs.
  • Treatment includes blood transfusions.
  • Types include 𝛃 thalassemia and 𝛂 thalassemia.
𝛃 Thalassemia
  • Synthesis of beta chains are defective or missing.
  • A defect or absence of beta-globin chain synthesis causes it.
  • Minor 𝛃 Thalassemia: Only 1 gene is defective
  • It is false that in 𝛃 Thalassemia Major that only one gene is defective because in 𝛃 Thalassemia Major, both genes are defective
  • Synthesized by 2 genes.
  • Means there are 2 types of 𝛃 thalassemia:
    • Minor: Only 1 gene is defective
    • Major: Both genes are defective
𝛂 Thalassemia
  • Synthesis of alpha chains is defective or missing.
  • Synthesized by 4 genes.
  • Types:
    • Silent Carrier: 1 gene is defective. This is a mild form of Alpha 𝛂 Thalassemia
    • It is false that Hydrops Fetalis results from one alpha gene that is defective because Hydrops Fetalis (the most severe form of Alpha Thalassemia) results from all four defective alpha genes and is fatal for the
    • 𝛂 thalassemia minor: 2 genes are defective
    • Hb H disease: 3 genes are defective
    • Hydrops fetalis: All 4 genes are defective causing Fetal death, since alpha chains are needed for HbF synthesis

Aplastic Anemia

  • A rare but serious blood disorder.
  • There is a decrease or failure in the production of all types of blood cells in the bone marrow.
  • Includes stem cells, RBCs, WBCs and platelets.
  • Pancytopenia results: a condition with reduced numbers of all three major blood cell types.
  • Causes include toxins, radiation therapy, and chemotherapy.
  • Treatment includes bone marrow transplant with immunosuppression.

Sickle Cell Anemia

  • Indicates abnormal hemoglobin (Hgb-S) present in RBCs, creating long, stiff, rod-like shaped RBCs.
  • Sickle RBCs rupture easily and can obstruct blood vessels.
  • The lifespan decreases to less than 20 days if a high hemolysis rate occurs against erythropoiesis, resulting in hemolytic anemia.
  • Low oxygen supply to the tissues results in tissue damage
  • It is an inherited condition
  • SCA genes grant resistance to malaria: heterozygotes are relatively protected against malaria death due to altered sickle cell plasma membrane causing potassium leakage, which kills malarial parasites.
  • Homozygous patients suffer from sickle cell anemia and are highly susceptible to malaria death.
  • Symptoms include Hand-foot syndrome in children with swelling of the feet and wrists
  • Pain in the limbs and back in the elderly.
  • Treatment includes analgesics, antibiotics, and blood transfusions.

Normal Hemoglobin Types

  • HbA/ HbA1: 95-98% for healthy adults
  • HbA2: 2-3% for healthy adults
  • HbF: 50-80% for newborns, gradually declines throughout life

Abnormal Hemoglobin Types

  • HbS: Mutations occurring in the beta globin chains (6th position).
  • Sixth position of beta chain glutamic acid is replaced by valine.
    • HbAS: A mutation in only 1 B globin chain, asymptomatic
    • Is a heterozygous mutation
    • Have normal life spans
    • HbSS: A mutation in both B globin chains.
    • Is a homozygous mutation showing normal symptoms of anemia

Polycythemia

  • Abnormal increase in circulating RBCs, indicated by a hematocrit over 65%.
  • Results in increased blood viscosity, increased BP, and thrombosis.
  • Piling up of too many RBCs trying to enter smaller blood vessels.

Additional Information

Infectious Mononucleosis

  • Caused by the Epstein-Barr Virus (EBV).
  • Commonly known as the "Kissing Virus" due to transmission through oral contact.
  • Named "mononucleosis" because it causes B lymphocytes to resemble monocytes.
  • There is no specific cure.
  • Common symptoms include:
    • Fatigue
    • Enlarged lymph nodes
    • Sore throat
  • High blood pressure is not a symptom.

Hemostasis

  • First response to a damaged blood vessel: vascular spasm.
  • Platelet plug formation is for temporarily sealing small blood vessel injuries.
  • Final result of the coagulation cascade: conversion of fibrinogen to fibrin.
  • The extrinsic pathway is activated by external trauma and the intrinsic pathway is triggered by internal vessel damage.

Substances that affect Hemostasis

  • Prostacyclin (PGI2) is a powerful inhibitor of platelet adhesion and release.
  • Antithrombin III (AT-III) blocks the action of factors II, IX, X, XI, and XII.
  • Protein C inactivates factors V and VIII and enhances the activity of plasminogen activators.
  • Alpha-2-macroglobulin inactivates thrombin and plasmin.
  • Alpha-1-antitrypsin inhibits factor XI.
  • Heparin combines with AT-III, increasing its effectiveness in blocking thrombin.
  • Prostacyclin (PGI2) is also known as Prostaglandin I2 (PGI2).
  • Prostacyclin is a naturally occurring molecule derived from arachidonic acid, a fatty acid present in cell membranes.
  • Prostacyclin is primarily synthesized by endothelial cells and WBCs, opposing the action of TXA2.
  • Prostacyclin has a potent vasodilatory effect, helping to relax and widen blood vessels, contributing to blood pressure regulation.
  • Prostacyclin inhibits platelet aggregation or clumping, helping prevent the formation of unwanted blood clots.
  • Prostacyclin is anti-inflammatory and involved in the modulation of the immune response.
  • Anticoagulants inhibit the formation or activity of blood clots.
  • Activated protein C (APC) inactivates clotting factors V & VIII.
  • Antithrombotics include anticoagulants and anti-platelets.
  • Anticoagulants slow down clotting, reduce fibrin formation, and reduce clot formation and growth.
  • Examples of anticoagulants: Heparin and Warfarin.
  • Anti-platelets prevent platelets from clumping and prevent clot formation.
  • Aspirin is an example of an anti-platelet.
  • Thrombolytic agents dissolve blood clots.
  • Streptokinase is an example of a thrombolytic agent.

Hemostatic Disorders

  • Thrombocytosis is an abnormal increase in the platelet count.
  • Thrombocytopenia is an abnormal decrease in the platelet count.
  • Hemophilia is a deficiency or absence of a blood clotting factor and is a sex-linked, inherited disorder.
  • The defective gene in hemophilia is carried on the X chromosome.
    • Hemophilia A: Deficiency of factor VIII.
    • Hemophilia B: Deficiency of factor IX.
    • Hemophilia C: Deficiency of factor XI.
  • Symptoms of Hemophilia include numerous bruises, deep muscle bleeding and joint bleeding.
  • Intravascular clots can be induced by roughened endothelial surfaces of blood vessels due to atherosclerosis, trauma, or infection which induce platelet adhesion.
    • Thrombus: A stationary clot that forms in a blood vessel.
    • Emboli: Dislodged and transported thrombus to other parts of the body, air bubbles, fat, debris from broken bones, tumor cells.
  • Von Willebrand’s Disease is a deficiency in VWF and Factor VIII and is the most common inherited bleeding disorder which leads to excessive bleeding.
  • Developing hemophilia C is possible but rare in those with with Von Willebrand’s Disease
  • Von Willebrand’s Disease affects males and females equally and there is a 50% chance of passing this gene on his/her child.
  • Multiple Myeloma is a cancerous disorder of plasma cells with an increased amount of plasma cells where abnormal plasma cells often form tumors in bones or soft tissues of the body.
  • Plasma cells develop from B cells, a type of WBC that is made in bone marrow.
  • Plasma cells make a different antibody to fight each type of bacteria or virus that enters the body to stop infections and diseases.
  • Abnormal production of plasma cells can form tumors in bones or soft tissues.

Fibrinolysis

  • It is a process in which blood clots are dissolved or broken down after they have served their purpose.
  • Plasminogen is activated into Plasmin.
  • Plasmin dissolves the clot by digesting fibrin threads & inactivating fibrinogen (CFI), prothrombin (CF II) & CF V, VIII, XII).

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